教員業績データベース |
|
論文種別 | 原著 |
言語種別 | 英語 |
査読の有無 | 査読あり |
表題 | Mitochondrial respiratory chain complex IV deficiency complicated with chronic intestinal pseudo-obstruction in a neonate. |
掲載誌名 | 正式名:Pediatrics international : official journal of the Japan Pediatric Society 略 称:Pediatr Int ISSNコード:1442-200X(Electronic)1328-8067(Linking) |
掲載区分 | 国外 |
巻・号・頁 | 58(7),pp.651-5 |
著者・共著者 | Hashimura Yuya, Morioka Ichiro, Hisamatsu Chieko, Yokoyama Naoki, Taniguchi-Ikeda Mariko, Yokozaki Hiroshi, Murayama Kei, Ohtake Akira, Itoh Kyoko, Takeshima Yasuhiro, Iijima Kazumoto |
発行年月 | 2016/07 |
概要 | A female infant born at 36 weeks gestational age with birthweight 2135 g, and who developed respiratory disorder, hyperlactacidemia and hypertrophic cardiomyopathy after birth, was admitted to hospital at 3 days of age. After admission, bilious emesis, abdominal distention, and passage disorder of the gastrointestinal tract were resistant to various drugs. Exploratory laparotomy was performed at 93 days of age, but no organic lesions were identified and normal Meissner/Auerbach nerve plexus was confirmed, which led to a clinical diagnosis of chronic intestinal pseudo-obstruction (CIPO). She was diagnosed with mitochondrial respiratory chain complex IV deficiency on histopathology of the abdominal rectus muscle and enzyme activity measurement. This is the first report of a neonate with mitochondrial respiratory chain complex deficiency with intractable CIPO. CIPO can occur in neonates with mitochondrial respiratory chain disorder, necessitating differential diagnosis from Hirschsprung disease. |
DOI | 10.1111/ped.12907 |
PMID | 27264907 |