Clinicopathological studies on the diagnosis, the treatment and the pathogenesis if pseudo-Hirschsprung's disease and related disorders

假性先天性巨结肠及相关疾病的诊断、治疗及发病机制的临床病理学研究

• 批准号: 03304037
• 负责人: OKAMOTO Eizo
• 金额: $ 7.94万
• 依托单位: Hyogo College of Medicine
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Co-operative Research (A)
• 财政年份: 1991
• 资助国家: 日本
• 起止时间: 1991 至 1993
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-03304037/
• 关键词: pseudo-Hirschsprung's disease; immaturity of ganglia; hypoganglionosis; oligoganglionosis; hypogenesis of ganglia; chronic idiopathic intestinal pseudo-obstruction; megacystis microcolon intestinal hypoperistalsis syndrome; Hirschsprung's disease /

On hundred and thirty cases congenital intestinal motor dysfunction were collocted by number of Grant-in Aid for Co-operative Research (A) (No.03304037) from the Ministry of Education, Science and Culture, Japan during the post 20 years. The disorder was defined as a congenital, non-mechanical obstruction of the intestine with the presence of intramural ganglia in the triminal rectum. Intramural ganglia were abnormal in 86 cases, normal in 37, and could not be determined in 8. Of those with abnormal intramural ganglia, 27 had immature ganglia, 29 had hypoganglionosis, 20 had both the immature ganglia and hypoganglionosis, 5 had neuronal intestinal dysplasia, and 2 had a segmental anomaly. Of those with a normal myenteric plexus, 15 had chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) and 11 had megacytis-microcolon intestinal hyperristalsis syndrome (MMIHS). Most cases congenital intestinal motor dysfunction without aganglionosis had normal acetylcholinesterase activity. While cases with normal intramural ganglia had normal rectoanal reflex, a significantly greater number of patients with hypoganglionosis or immature ganglionosis locked rectoanal reflex. While patients with immature ganglionosis had good prognosis, patients with hypoganglionosis, CIIPS and MMIHS had poor prognosis. We define pseudo-Hirschsprung's disease as abnormal group of intramural ganglia among cases with congential intestinal motor dysfunction. These findings indicate that psudo-Hirschsprung's disease remains a serious disease of childhood.

根据日本文部科学省合作研究资助（A）号（No.03304037），收集了近20年来130例先天性肠运动功能障碍患者。该疾病被定义为先天性，非机械性肠梗阻，并在直肠三尖瓣内存在壁内神经节。壁内神经节异常86例，正常37例，不能确定8例。在壁内神经节异常的患者中，27例为未成熟神经节，29例为神经节发育不良，20例同时为未成熟神经节和神经节发育不良，5例为肠神经元发育不良，2例为节段性异常。在肌间神经丛正常的患者中，15例患有慢性特发性假性肠梗阻综合征（CIPS），11例患有巨细胞-小结肠肠增生综合征（MMIHS）。无神经节细胞缺乏症的先天性肠运动功能障碍者乙酰胆碱酯酶活性大多正常。而正常的壁内神经节的情况下，正常的直肠肛门反射，一个显着更多的患者与hypoganglionosis或不成熟的神经节病锁定直肠肛门反射。未成熟神经节细胞增生症患者预后较好，神经节细胞减少症、CIIPS和MMIHS患者预后较差。我们将假性先天性巨结肠定义为先天性肠运动功能障碍病例中的肠壁内神经节异常组。这些发现表明，假先天性巨结肠症仍然是一个严重的疾病的儿童。

项目成果

Shimotake T, Iwai N, et al.: "Biliary tract complications in patients with hypoganglionosis and chronic idiopathic intestinal pseudo-obstruction syndrome." J Pediatr Surg. 28. 189-192 (1993)

Shimotake T、Iwai N 等人：“神经节减退症和慢性特发性肠假性梗阻综合征患者的胆道并发症。”

Yokoyama J, et al.: "Immunohistochemical evaluations for muscle layrs and nerve fibers of the bowels with neurogenic disorders." XXI International Congress of Neurovegetative Research, Neuronal Regulation of Visceral Functions : Physiology and Physiopatho

Yokoyama J 等人：“对患有神经源性疾病的肠道肌肉层和神经纤维进行免疫组织化学评估。”

Taguchi T, Suita S, et al.: "Chronological changes in the pathological findings of myenteric plexus in allied disorders of Hirschsprung's disease." XXXIst Annual International Congress of British Association of Pediatric Surgeons (Rotterdam). (1994)

Taguchi T、Suita S 等人：“先天性巨结肠相关疾病中肌间丛病理结果的时间变化。”

富本 喜文: "腸管壁内神経細胞の未熟性が原因と考えられるイレウス症例の検討" 第20回日本腹部救急医学会(1993年3月発表予定). (3月). (1993)

Yoshifumi Tomimoto：“被认为是由肠壁神经细胞不成熟引起的肠梗阻病例的研究”，第 20 届日本腹部急救医学会（计划于 1993 年 3 月发表）（1993 年 3 月）。

A.Toyosaka: "A histopathological study of hypoganglionosis of the intestine:its pathophysiology and the treatment." New Trends in Autonomic Nervous System Research(Published by Elsevier Science Publishers B.V.). 289 (1991)

A.Toyosaka：“肠道神经节减退症的组织病理学研究：其病理生理学和治疗。”