Journal of the Japanese Society of Pediatric Surgeons
Online ISSN : 2187-4247
Print ISSN : 0288-609X
ISSN-L : 0288-609X
Volume 28, Issue 4
Displaying 1-50 of 84 articles from this issue
  • Article type: Cover
    1992 Volume 28 Issue 4 Pages Cover1-
    Published: June 20, 1992
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  • Article type: Cover
    1992 Volume 28 Issue 4 Pages Cover2-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App1-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App2-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App3-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App4-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App5-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App6-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App7-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App8-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App9-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App10-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App11-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App12-
    Published: June 20, 1992
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App13-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App14-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App15-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App16-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App17-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App18-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App19-
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App20-
    Published: June 20, 1992
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  • Article type: Appendix
    1992 Volume 28 Issue 4 Pages App21-
    Published: June 20, 1992
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  • Article type: Index
    1992 Volume 28 Issue 4 Pages Toc1-
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  • [in Japanese]
    Article type: Article
    1992 Volume 28 Issue 4 Pages 793-
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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  • Makoto Iwafuchi
    Article type: Article
    1992 Volume 28 Issue 4 Pages 794-797
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Twenty nine years have elapsed since Japnaese society of pediatric surgeons (JSPS) was organized in 1964. At the present time, JSPS with about 3,600 participants, is one of the main societies with a deep relationship to Japan Surgical Society. Furthermore, JSPS has organized the Board on Speciality Certification in Pediatric Surgery since 1979, with 135 certified instructors on pediatric surgical training,and with 211 certified competences. 1. Specificity of pediatric surgery is thought to be as follows : 1) Surgery of newborns, infants and children 2) Plastic surgery aiming to improve the dysfunction of organs 3) Surgery of pediatric solid malignant tumors 4) Surgery of pediatric surgical disease, regardless of patient's age 5) Special surgery different from general surgery 2 . Future problems of pediatric surgery may be as follows. 1) Low cost performance of surgery in pediatric patients 2) Decrease of child birth in recent days 3) Risk of fragmentation of surgery 4) Shortage of man power from view point of research From the aspect of social demand, pediatric surgeons seem to have to do training of general surgery in addition to pediatric surgery.
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  • Ryoji Ohi
    Article type: Article
    1992 Volume 28 Issue 4 Pages 798-802
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Pediatric surgery in Japan has achieved gradual recognition as one of the clinical specialties by the tremendous efforts of senior pediatric surgeons. However, it is a fact that there are currently so many difficult problems in the fields of education, training program, research investigations and clinical practice of pediatric surgery in our country. Although there is no doubt that pediatric surgical patients should be cared by certified pediatric sugeons, the author believes that pediatric surgeon must train as a general surgeon and work closely with general surgeons and with other related specialties. On the other hand, we should endeavor to increase the number of medical educational institutions, finacial support for research works and medical facilities of pediatric surgery in our country. It is also important to prevent unnecessary fragmentation of pediatric surgical care in the future.
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  • Tetsuo Kato
    Article type: Article
    1992 Volume 28 Issue 4 Pages 803-807
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Issues in pediatric surgery in Japan, including clinical medicine, research, education, ethics, patient health, medical expenses, and malpractice suits were discussed from the standpoint of a pediatric surgeon working at a university hospital. Various aspects of each issue were explored, and strategies of each problem area were proposed. The four starategies particularly emphasized were advancement of clinical field in peadiatric surgery, development of a new mass screeinig program to detect malignant tumors and active participation in the mass screening team, clarification of the etiology of biliary atresia and development of an effective treatment, and advancement of research by a newlyformed project team whose members represent as many institutions in Japan as possible.
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  • Chikara Tsugawa
    Article type: Article
    1992 Volume 28 Issue 4 Pages 808-811
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    The issue of pediatric surgical manpower is critical to the long-range planning for national health care in childre. In several countries where pediatiric surgery is well established, one pediatric surgeon per half million population is recognized as a reasonable number. Board certified pediatric surgeons at present in Japan is 211 and this number/population ratio seems to be reasonable. To estimnate the proper pediatric surgical manpower in the future, a computer analysis was made to project numbers of pediatric surgeon in pediatric population until the year 2020 with differing level of trainee output. The study indicates that (1) if current trends of increasing ratio continue, the number of pediatric sugeon after the year of 2000 is extraordinarily large. (2) individuals entering the system every year should be restricted to 10-13 to allow for modest growth of the speciality.
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  • Hiroshi Akiyama, [in Japanese], [in Japanese], [in Japanese]
    Article type: Article
    1992 Volume 28 Issue 4 Pages 812-815
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Kagoshima prefecture is located most southern part of Japan including many isolated island. In this are, two million population and twenty thousand birth number in each year was found and about fifty cases in neonatal surgical patients were treated in each year. In this peper, many future problems of pediatric surgery including pre and postgraduated education, and clinical and research studies were discussed.
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  • Morihiro Saeki, Toshiro Honna, Miwako Nakano, Kazuhiko Hagane, Yoshiyu ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 816-819
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    The National Children's Hospital (NCH) was founded in 1965 as the first general children's hospital in Japan. It has practical functions only for infants and children. Another Jaspanese children's hospitals have almost the same condition as NCH, which lacks for functions of dealing older age patients. However, the number of admisions of the patients, who were treated in the early years and have reached more than 16 years old, is rapidly increasing. Environements at childre's hospital are not always covenient for the older patients not only in medical aspects but also in social aspects. Girls who underwent pelvic surgery or who have impaired hepatic, renal, or cardiac function are coming to their reproductible age. Pregnancy and delivery in such patients may be anticipated seriously risky. Pediatric surgeon, pediatrician, and obstetrician should be closely connected to treat high-risk pregnancy. Furthermore, children's hospitals should respond to maternal transportaion in cases of antenatal diagnosis. A faculity for the adult pracitce, incluidng obstetrics and gynecology, has become necessary in old children's hospitals in Japan.
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  • Sachiyo Suita
    Article type: Article
    1992 Volume 28 Issue 4 Pages 820-824
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    The pioneering work of a few surgeons in 1960s made a small bud of pediatric surgery in Japan, which grew rapidly afterward and by the 1980s pediatric surgery was recognized as a specialtiy and independent pediatric surgical departments were established in several universities and childre's hospitals. A number of difficult problems, however, are currently confronting the field of pediatric surgery, such as a bottle-neck of pediatric surgeons as the results of overproduciton of trained pediatric surgeons, decreased number of patients due to decreased birth rate, and the rapid development of the organ specialties ; i. e., urology, neurologic-, plastic-, micro-surgery, transplantation, traumatilolgy and oncologist invading into the territory of pediatric surgery. These problems were reviewed and some ways of overcoming these problems were proposed with reference to the views from 16 affiriated pediatric surgeons from 15 countireis in the world.
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  • Kinji Nagashima
    Article type: Article
    1992 Volume 28 Issue 4 Pages 825-829
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Pediatric surgery in Japan has made remarkable progress since its birth in early 1960s. But in the developing countries, where there are many problems such as a explosive increase in the population and very low family income, many children could not and still can not receive appropriate treatment. World organizations such as World Federation of Associations of Pediatric Surgeons, Surgical Aid to Children of the World and Japan International Cooperation Agency have done and are doing its best to save children with surgically curable diseases, but not sufficient to resolve problems. Now Japanese Society of Pediatric Surgeons should play an important role in surgical aid to save handicapped children in the developing countries.
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  • [in Japanese], [in Japanese]
    Article type: Article
    1992 Volume 28 Issue 4 Pages 830-834
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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  • Yoshihiko Kishida
    Article type: Article
    1992 Volume 28 Issue 4 Pages 835-842
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Experimental studies of HFO were performed on rabbits to determine the proper setting of the MERA BMO Hummingbird 20N, which provided the best respiratory and circuratory support. After they had been anesthetized, animals were placed on the Hummingbird. PaO_2, PaCO_2, BP, CVP, HRand ITP were determined at different SV, MAP, and Fq. The following results were obtained ; 1. Best respiratory and circulatory data were obtained at 5ml/kg SV, 10cmH_2O MAP, and 15Hz Fq. HFO itself did not influence the mean blood pressure or the heart rate. 2. In an animal model of congenital diaphragmatic hernia produced by inflating a balloon in the thoracic cavity, HFO removed carbon dioxide more effectively than a conventional ventilator. 3. Since HFO did not ventilate the lung well through a narrow trachea less than 2.0mm I. D., an endotracheal tube used for HFO should be at least 3.0mm I. D.
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  • Shigeru Ueno, Haruo Ishida, Akira Hayashi, Shoichiro Kamagata, Shinich ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 843-850
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Management of the fecal incontinence is most important aspect of postoperative care of the patients with high-type imperaforate anus. In our institution, patients with the anomalies are treated with the enema progarm after the definitive surgery, under which they are expected to defecate habitually by the everyday enema unitl they achieve satisfactory bowel control. Effect of the habitual enema on the postoperative fecal continence was investigated on 30 patients over 4 years of age, who were operated during the past 20 years. They were evaluated clinically by using the scoring system proposed by the Japnaese Society of Pediatric Surgeons. Manometric studies and radiological examinations were also done upon 25 of them to analyse their local function and structure. Children with the malformation tend to have low scores on fecal sensation in their first decade of life and most of them (87%) are under the enema program. More than half of those under the program (58%) are free from the incontinent state regardless of their sense of defecation. Involuntary fecal excretions are avoided unless the stool is loose, which indicates that the habitual enema is effective for them to live without being frustrated by soilings. The patients for which the program is effective tend to have better objective scores than those whose everyday enemas are less effective. After the long-term habitual enema, most adolescent patients can sense the defecation and are in controllable states even if the objective findings indicate poor local functional state.
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  • Masafumi Naito, Makoto Iwafuchi, Yoshihiro Ohsawa, Masanori Uchiyama, ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 851-855
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    In order to evaluate the stability of DNA histograms obtained from patients with neuroblastoma, 512 samples from 62 patients were examined. In almost all patients, every DNA histogram from different tumor portions had the same DNA index. Heterogeneity was detected only in 3 patients. To evaluate whether DNA ploidy has the potential to change its type with progression or not, we investigated DNA histograms obtained at different periods from the same patients. No change of DNA ploidy was detected in this study. In conclusion, DNA histogram is considered to be stable in neuroblastoma.
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  • Takao Morita, Kiyoshi Ishida
    Article type: Article
    1992 Volume 28 Issue 4 Pages 856-864
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    In order to investigate the role of prolactin in the growth of intestinal mucosa, we experimentally produced endocrinological enviroments of prolactin secretion in hyperprolactinemia and hypoprolactinemia by administration of bromocriptine or perphenazine to mice. The results were as follows : 1) These administrations induced no change in the gastrointestinal muscle coats as opposed to in its mucosa. 2) The small bowel mucosa tended to grow to the lagest in the hyperprolactinemia group, followed by the controls and the hypoprolactinemia group in that order (p < 0.05). 3) There was no apparent correlation between prolactin secretion and gastric and large bowel mucosa. We conclude that prolactin may be trophic to the intestinal mucosa in mice, suggesting a growth promoting role of prolactin on the mid gut epithelium in the fetal stage.
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  • Miyuki Kohno, Teruho Kaiimoto
    Article type: Article
    1992 Volume 28 Issue 4 Pages 865-868
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Inguinal and scrotal temperatures were measured by thermometer in 45 children undergoing inguinal surgical procedures with general anesthesia. Temperature measurements were taken from the skin surface and subcutis of the inguinal, suprascrotal and scrotal areas. Intrascrotal temperature inside the dartos muscle was also measured at the scrotum. Temperatures on the unaffected side were examined separately from temperatures on the side of the lesion. Temperatures in the cryptorchid side were also compared with that on the unaffected side. The temperature difference between the subcutaneous area and inside the dartos muscle in the scrotal region for the unaffected side was 0.9 °C. This difference was statisfically significant. This finding suggests that a testis enclosed in the dartos pouch from orchidopexy lies in a lower environmental temperature than the unaffected testis. But we think this temperature difference has no effect on the function of the testis. Intrascotal temperature on the cryptorchid side was slightly higher than that on the unaffected side. We think it is necessary to examine the effect the dartos pouch has on the testis in orchidopexy.
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  • Yutaka Kanamori, Kohei Hashizume, Hideo Kawarasaki, Tadashi Iwanaka, T ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 869-874
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Eleven cases of limbo-sacral lipomas with spinal dysraphism were treated in our institute from 1976 to 1991.Their clinical features, treatment and neurological prognosis were analysed retrospectively. Their chief complaints were lumbo-sacral lipomas in nine cases and neurological abnormalities in two cases. Ten out of eleven cases were first presented to our department at the age under two. Surgical treatment were done in nine cases. In six of them only partial resection of lipomas was performed without opening the dura mater. In remining 3 cases the dura mater was opened and the spinal cord and nerves were dissected from the lipoma. Neurological deficits remained in two cases and new neurological deficits appeared in three cases afther the first operations In inadequete operetions or in those whose operations were done after the appearance of neurological disorder the neurological prognosis was not satisfactory. We are now going to resect the lipomas as completely as possible using laser knife under neurological monitoring before neurological symptoms appear in the coming patients.
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  • Shoichiro Kamagata, Haruo Ishida, Akira Hayashi, Shigeru Ueno, Shinich ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 875-880
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Thirty three cases of GER in newborn infants were reviewed. Manometric study (23 cases), 24 hour pH monitoring (23 cases) and endoscopy (33 cases) were done at admission and repeated three weeks later. Twenty seven patients were followed from one month to 8 years. Male and female ratio was 17 : 16, 27 cases (80%) had symptoms before 72 hours after birth. Severe esophagitis with ulcer and erosion was found in 25 cases and equally frequent with or without hiatus hernia (HH). But pH score was significantly higher in patient with HH. Twelve cases of GER without HH healed in one month and no one needed surgery. In GER with HH, vomiting disappeared between 3 and 12 months (mean 5.2 months) and reflux under fluoroscopy disappeared between 3 and 14 months (mean 7.6 months). One patient with HH underwent surgery because of failure to thrive and persisting esophagitis. The prognosis of GER in newborn period is much better than expected, and medical therapy is tried while the symptoms and results of examination are improving.
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  • Yasuo Ito, Yuji Nirasawa, Kyoko Satsumabayashi, Hiroyuki Tanaka, Akiku ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 881-886
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Human leucocyte antigens (HLA) were examined in 8 families with hereditary imperforate anus and 35 patients of imperforate anus without a family history. The results were compared with those of 310 healthy Japanese controls. In 4 families, in which 2 generations were affected, all affected members had a common haplotype, whereas healthy members did not have the haplotype. This result indicates that the disease-causing gene was transmitted with HLA in a dominant form. In 3 families, in which only siblings were affected, all affected siblings had a common haplotype. In 1 family, however, affected siblings did not have a common haplotype, which may indicate heterogeneity of the anomaly. On the other hand, HLA haplotype of AW33-C--B44 was observed in 3 families with heredity (38%), while the frequency of this haplotype in Japanese population is only 6%. Furthermore, HLA-AW33 was associated with affected members of 4 families (50%, control 16%). These associations of HLA with hereditary imperforate anus suggest that the disease-causing gene has closer linkage with HLA-A antigens. AW33 and B44 were not frequent in non-hereditary imperforate anus. Frequencies of A2 (P < 0.025), CW3 (P < 0.025), DR4 (P < 0.05), and DQW3 (P < 0.05) were higher in this group than those in controls. B51, on the contrary, was less frequent (P < 0.05). DR4 showed closer association with the high type of imperforate anus (n = 20, P < 0.05), while DR2 with the low type (n = 15, P < 0.05). Immune response may have some influence on deciding the types of imperforate anus.
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  • Kosaku Maeda, Tetsuya lenaga, Tetsuo Yamamoto
    Article type: Article
    1992 Volume 28 Issue 4 Pages 887-891
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    From September 1990 through September 1991, seven patients underwent 10 CO_2 laser procedures for treatment of airway lesions. In four patients via bronchoscopy and in three patients via operation field. Ages ranged from 6 months to 8 years. Diagnoses included '・ Acquired subglottic stenosis (6) and Tracheal bleeding (l). Most lesions responded to only one to two laser treatments. No major complication occurred secondary to laser surgery. Significant benefits, such as minimal postoperative edema and scarring, and minimum risk associated with CO_2 laser surgery were recognized. It may play an important part in treating obstructive airway lesion of infants and children.
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  • Hiroyoshi Komai, Yasuaki Naito, Keiichi Fujiwara, Yusaku Takagaki, Hir ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 892-896
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    An 8-year-old boy was suffered from progressive subcutaneous and mediastinal emphysema following blunt chest trauma. Emergency bronchoscopy revealed tracheal injury just above the carina. The tear of membranous portion was closed with nonabsorbable monofilament sutures through fouth intrecostal thoracotomy. Because the tissue around the injury was weak and massive air leak remained, the suture site was covered with the latissimus dorsi muscle as a pedicle flap. Postoperative clinical course was excellent and the chest CT and bronchoscopy demonstrated no findings of tracheal stenosis at 6 months after the operation. We concluded that this flap method is efective to repair the tracheal injury, especially in cases withe weak tissues.
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  • Masaki Nio, Toshihiro Muraji, Hiroaki Ohue, Masato Sato, Eiji Nishijim ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 897-901
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Two infants with syndromatic paucity of interlobular bile ducts associated with concurrent extrahepatic biliary obstruciton are reported. Agenesis of extrahepatic bile ducts was documented by surgical exploration of the porta hepatis in both patients during hepatic portoenterostomy at the age af 2 and 3 months. They were suspected of syndromatic paucity of interlobular bile ducts because their chest films showed butterfly-like vertebral anomalies and additionally because a heart murmur was audible in one. Hepatic biopsy specimens revealed paucity of interlobular bile ducts, and minimal portal fibrosis. As these findings were characteristic of syndromatic paucity of interlobular bile ducts, both infants were diagnosed as having paucity as well as extrahepatic biliary atresia. Operative outcome was poor in terms of postoperative extended bile excretion. However both are still living at one and 4 ytears 5 months of age. Although it was hoped that hepatic portoenterostomy might have effectively prolonged their lives, retrospectively this procedure can not be considered definitive. In conclusion, it is quite likely that some etiologic factor which is predisposing to paucity of interlobular bile ducts in Alagille's syndrome affected extrahepatic as well as intrahepatic biliary tracts in certain cases.
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  • Takeshi Yamanouch, Akira Nagasaki, Hiroshi Ohgami, Takashi Kawanami, M ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 902-906
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    We reported a case of 12-year-old boy with thymolipoma. He was presented to our hospital with his abnormal chest X-ray finding with no symptom. Although his chest X-ray initialy suggested cardiomegaly, other examinations such as ultrasound sonography, computed tomography (CT) and magnetic resonance imaging (MRl) indicated a mediastinal tumor originated from thymus mixed with fat tissue. At thoracotomy the tumor was resected completely in four pieces. Total weight of the tumor was 746g. The diagnosis was made histopathologically. Thymolipma is a most likely diagnositic when we find a huge mediastinal tumor in a yong adult patient.
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  • Yuhei Goto, Yasuaki Naito, Keiichi Fujiwara, Shinichi Higashiue, Yusak ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 907-913
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    A 4-month-old girl with a rare type of double outlet right ventricle (DORV) {situs solitus, D-loop, L-transposition ; S. D. L.} associated with subpulmonic ventricular septal defect (VSD), single coronary arterey, patent ductus arteriosus, left juxtaposition of the atrial appendages and right aortic arch underwent urgent corrective surgery due to sever heart failure. Total correction was as follows. Intraventricular rerouting from the enlarged VSD to the aorta was performed using a Dacron patch. The right ventricular outflow tract was narrowed by this intraventricular tunnel, so an additional right ventricular outflow tract was reconstructed utilizing with an external conduit containing the pericardial bicuspid valves to avoid injury of the right coronary artery across the right ventricular outflow. Postoperative hemodynamics were satisfactory and stable, but she died suddenly of hypoxia on the 2nd postoperative day. The procedure reported on here may be an alternative for correction of this anomaly in an infant. The operative measures for DORV {S. D. L.} were reviewed and discussed.
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  • Takeo Yonekura, Noboru Nagahara, Tetsuro Nakamura, Akira Kashiwai, Har ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 914-921
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    Two cases of a four-month-old boy with malignant rhabdoid tumor of the kidney (MRTK) and a one and a half-year-old girl with clear cell sarcoma of the kidney (CCSK) are presented. They were treated by surgery, radiation therapy and chemotherapy according to the regimen-DD of the Third National Wilms' Tumor Study. They are alive without tumors after 38 months and 35 months after therapy, respectively. Twenty-two CCSK patients and 14 MRTK patients have been reported in Japanese literature. Seven CCSK and 3 MRTK patients stayed alive without tumors for more than 2 years after diagnosis.
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  • Tetsuya lenaga, Kosaku Maeda, Tetsuo Yamamoto
    Article type: Article
    1992 Volume 28 Issue 4 Pages 922-925
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
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    A very rare case of 48, XXYY Klinefelter's syndrome was found at repair of inguinal hernia. A 8-year-old female had bilateral groin tumors suspected to be herniated ovaries. At the time of herniorrhaphy, the tumor seemed to be testis. Biopsy of the tumor was performed and confirmed it hypogonadal testis. We removed the bilateral testes at the second operation. Cytogenetic study after these operations revealed a 48, XXYY karyotype ; a very rare Klinefelter's syndrome varient. Phenotype of this syndrome commonly showed male type of external genitalia. However, in this case, the patient had a female phenotype with absence of the uterus and oviduct, and a short vagina ending blindly. It is considered that this case might be 48, XXYY Klinefelter's syndrome associated with testicular feminization syndrome.
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  • Hirofumi Nakamura, Michio Kaneko, Haruo Okawa, Gohei Ochi, Mayumi Iwak ...
    Article type: Article
    1992 Volume 28 Issue 4 Pages 926-931
    Published: June 20, 1992
    Released on J-STAGE: January 01, 2017
    JOURNAL FREE ACCESS
    Intracaval involvement of Wilms' tumor is a rare condition. This is a report of a 7 -year-old girl with intracaval involvement of Wilms' tumor. The tumor originated in the left kidney and the tumor thrombus extended into the retrohepatic inferior vena cava through the renal vein. Total excision (kidney and thrombus) was accomplished safely by venacavotomy with proximal and distal vascular control. The patient is in good health with postoperative radiation and chemotherapy. Major intraoperative hemorrhage, bowel obstruction, occlusion of the inferior vena cava after removal of the thrombus, nonlethal intraoperative cardiac arrest, and pulmonary embolism have been reported as complications of surgical management. Surgical management of Wilms' tumor extending into the inferior vena cava is a daunting surgical challenge. Careful preoperative evaluation of the tumor thrombus to define the cephalad limit of venous extension and appropriate vascular control are important to avoid complications.
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