TOTAL AGANGLIONOSIS OF THE COLON (HIRSCHSPRUNG'S DISEASE) and CONGENITAL FAILURE OF AUTOMATIC CONTROL OF VENTILATION (ONDINE'S CURSE)
TOTAL AGANGLIONOSIS OF THE COLON (HIRSCHSPRUNG'S DISEASE) and CONGENITAL FAILURE OF AUTOMATIC CONTROL OF VENTILATION (ONDINE'S CURSE)
复制标题
结肠完全无神经节细胞增多症（先天性巨结肠症）和先天性通气自动控制失败（翁丁诅咒）
DOI：
10.1111/j.1651-2227.1981.tb07184.x
复制
发表时间：
1981
期刊：
影响因子：
--
通讯作者：
W. Lambrecht
中科院分区：
文献类型：
--
作者：
M. Stern;H. Hellwege;L. Grävinghoff;W. Lambrecht
抽象。斯特恩，M.，赫尔韦格湾H、格雷温霍夫湖和Lambrecht，W.（联邦德国汉堡Eppendorf大学医院儿科和外科）。结肠完全无神经节细胞症（先天性巨结肠症）和先天性自动通气控制失败（Ondine's curse）。Acta Paediatr Scand，70：121，1981.-在一名男孩中观察到新生儿期结肠完全无神经节细胞症伴小肠梗阻，并伴有需要持续机械通气的先天性肺泡通气不足。病人死于急性脱水肠炎，15个月大，长期后全切除无神经节肠已进行。新生儿期发现肺动脉高压。右心室心肌肥厚进行性。这是第四例报告的脑干和胃肠道神经细胞功能缺陷的组合。
Abstract. Stern, M., Hellwege, H. H., Grävinghoff, L. and Lambrecht, W. (Department of Paediatrics and Department of Surgery, University Hospital Eppendorf, Hamburg, Federal Republic of Germany). Total aganglionosis of the colon (Hirschsprung's disease) and congenital failure of automatic control of ventilation (Ondine's curse). Acta Paediatr Scand, 70:121, 1981.–Total aganglionosis of the colon presenting with small intestinal obstruction in the neonatal period was observed in combination with congenital alveolar hypoventilation requiring continuous mechanical ventilation in a boy. The patient died aged 15 months from acute dehydration due to enteritis, long after total resection of the aganglionic bowel had been performed. Pulmonary hypertension was found in the newborn period. There was progressive right ventricular myocardial hypertrophy. This is the fourth case reported with a combination of defects involving nerve cell function of the brain stem and gastrointestinal tract.