A Case of Hirschsprung's Disease Associated With Congenital Central Hypoventilation Syndrome (CCHS) Suffered From Cardiac Arrest Due to Bradyarrythmia
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- Shimura Tadanobu
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Inoue Mikihiro
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Ide Shozo
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Hashimoto Kiyoshi
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Matsushita Kohei
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Koike Yuhki
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Uchida Keiichi
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
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- Kusunoki Masato
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduated School of Medicine
Bibliographic Information
- Other Title
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- 徐脈性不整脈により心停止を来たした先天性中枢性肺胞低換気症候群(CCHS)合併Hirschsprung病(Haddad症候群)の1例
- 症例報告 徐脈性不整脈により心停止を来たした先天性中枢性肺胞低換気症候群(CCHS)合併Hirschsprung病(Haddad症候群)の1例
- ショウレイ ホウコク ジョミャクセイ フセイミャク ニ ヨリ シン テイシ オ キタ シタ センテンセイ チュウスウセイ ハイホウテイカンキ ショウコウグン(CCHS)ガッペイ Hirschsprungビョウ(Haddad ショウコウグン)ノ 1レイ
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Abstract
This is a case report of Hirschsprung's disease associated with congenital central hypoventilation syndrome (CCHS) suffered from cardiac arrest due to bradyarrythmia. A 9-day-old male was transferred to our hospital because of recurrent apnea, abdominal distention, and bilious vomiting probably due to a small intestinal obstruction. An emergency laparotomy performed revealed caliber change at 15 cm proximal from the cecum. Ileostomy and appendectomy were then performed. He was pathologically diagnosed with total colon aganglionosis. After the operation, his apnea did not improve. Genetic testing showed PHOX2B mutation and polyalanine expansions; the diagnosis of CCHS was confirmed. He had previously suffered from bradyarrythmia several times, and cardiac arrest occured at the age of 112 days. However, he recovered by cardiopulmonary resuscitation and therapeutic hypothermia without neurological damage. He is now 11 months old, and he has been staying in our hospital since admission.
Journal
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- Journal of the Japanese Society of Pediatric Surgeons
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Journal of the Japanese Society of Pediatric Surgeons 48 (7), 1029-1032, 2012
The Japanese Society of Pediatric Surgeons
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Details 詳細情報について
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- CRID
- 1390282679809828480
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- NII Article ID
- 110009562247
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- NII Book ID
- AN00192281
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- ISSN
- 21874247
- 0288609X
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- NDL BIB ID
- 024167602
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL
- CiNii Articles
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- Abstract License Flag
- Disallowed