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- FURUTA Mutsuhiro
- Kyoto National Hospital
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- ITO Tsuyoshi
- Kyoto National Hospital
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- OBARA Akiko
- Kyoto National Hospital
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- KANEOKA Masaki
- Kyoto National Hospital
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- SOKAI Katsuhiko
- Kyoto National Hospital
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- KITAICHI Masanori
- Kyoto National Hospital
Bibliographic Information
- Other Title
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- Goldenhar症候群
- Goldenhar ショウコウグン
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Description
Goldenhar syndrome, known as oculoauriculo vertebral dysplasia, is characterized by malformations of eyes, ears and vertebral bones and sometimes associated with unilateral facial hypoplasia. However, we have no criteria at present to distinguish this syndrome from other similar diseases or syndrome including mandibulofaial dysostosis, hemifacial microsomia etc. We think it better to call this so-called Goldenhar syndrome.<br>As there have been almost no reports about pathological findings of this syndrome, we can't be sure about the pathological features, but the common histopathological findings of our two cases are renal cortical cysts, enlargement of Graaf ian follicle, increased and enlarged islets of pancreas, and involution of thymus. These findings are the features of serious congenital malformations including central nervous system. The etiology of so-called Goldenhar syndrome is not known, but we postulate certain causes that would make these malformations of eyes, ears, vertebral bones, heart, both extremities, facial bones etc., and this would occur at the early stage of four weeks gestation.<br>Associated malformations such as anterior chest wall heterotopic exostosis, situs inver-sus totalis observed in our first case, and esophagotracheal fistula observed in the second case, have not been reported. But the dermoid cyst of the Pith ventricle has been pre-viously noted in the literature. Thus, the presence of dermoid cyst in our second case of three-days-old and 1360g of birth weight is very interesting.
Journal
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- Japanese Journal of National Medical Services
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Japanese Journal of National Medical Services 37 (5), 463-468, 1983
Japanese Society of National Medical Services
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Keywords
Details 詳細情報について
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- CRID
- 1390282681291207680
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- NII Article ID
- 130004313241
- 40000144415
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- NII Book ID
- AN00017113
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- ISSN
- 18848729
- 00211699
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- NDL BIB ID
- 2630683
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL Search
- CiNii Articles
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- Abstract License Flag
- Disallowed