Ohkubo Hidenori, Inoh Yumi, Fuyuki Akiko, Nakajima Atsushi
Nihon Rinsho. 2015 May;73(5):875-83.
Chronic intestinal pseudo-obstruction(CIPO) is a rare severe digestive disease in which clinical symptoms of intestinal obstruction appear without any mechanical cause. Pathophysiologically, CIPO shows ineffective intestinal propulsion due to an impairment of intestinal smooth muscle, enteric nervous system, and interstitial cells of Cajal(ICC). Sustained increased intra-bowel pressure often causes small intestinal malabsorption and bacterial translocation, and leads to malnutrition and blood stream infection (sepsis). Key points of the medical approach for CIPO are to improve nutritional status and reduce abdominal symptoms. Dietary cure and defecation control are the main options in mild cases, whereas home-parenteral-nutrition(HPN) and decompression therapy are often needed in severe cases. Stimulant laxatives, prokinetics and herbal medicine are usually used but often in fail. Percutaneous endoscopic gastrojejunostomy(PEG-J) tube may be burdenless compared to conventional ileus tube. Most important points in the management of this disease are to make a correct diagnosis as early as possible and avoid unnecessary surgery. However, no clear diagnostic criteria have been established so far. Manometry, scintigraphy, and full-thickness biopsy are the major examination for the CIPO diagnosis in the Western countries; however these specialized examinations are not popular in Japan. Therefore the Research Group(chief investigator, Atsushi Nakajima) proposed Japanese diagnostic criteria in 2009 to facilitate the diagnosis of this rare disease by the general physician. In 2013, we have reported that cine-MRI is a non-invasive diagnostic method for CIPO. Although further data are eagerly awaited, it can become a promising diagnostic tool in CIPO patients. Furthermore the Japanese criteria have been revised, and in 2014, the comprehensive criteria from a child to an adult have been devised. In 2015, CIPO is newly certified as Specified Rare and Intractable Disease which is subsidized from public expense, in Japan. In the future, the diagnostic criteria should be appropriately modified by consultation with additional researchers to make them more practical and internationally applicable.
慢性假性肠梗阻（CIPO）是一种罕见的严重消化系统疾病，其肠梗阻的临床症状在没有任何机械性病因的情况下出现。在病理生理学上，CIPO表现为由于肠道平滑肌、肠神经系统和 Cajal 间质细胞（ICC）受损而导致的肠道推进无效。持续升高的肠内压常导致小肠吸收不良和细菌易位，并导致营养不良和血流感染（败血症）。CIPO 的医学治疗要点是改善营养状况和减轻腹部症状。轻度病例的主要选择是饮食治疗和排便控制，而重度病例通常需要家庭肠外营养（HPN）和减压治疗。通常使用刺激性泻药、促动力药和草药，但往往效果不佳。与传统的肠梗阻导管相比，经皮内镜胃空肠造口术（PEG-J）导管可能负担较小。该疾病管理中最重要的要点是尽早做出正确诊断并避免不必要的手术。然而，到目前为止尚未建立明确的诊断标准。测压、闪烁扫描和全层活检是西方国家 CIPO 诊断的主要检查方法；然而，这些专业检查在日本并不普及。因此，研究小组（首席研究员中岛敦史）于 2009 年提出了日本诊断标准，以方便普通医生对这种罕见疾病进行诊断。2013 年，我们报告电影磁共振成像（cine-MRI）是一种用于 CIPO 的非侵入性诊断方法。尽管急切期待更多数据，但它可能成为 CIPO 患者有前景的诊断工具。此外，日本标准已修订，2014 年制定了从儿童到成人的综合标准。2015 年，CIPO 在日本新被认证为特定罕见难治性疾病并由公费补贴。未来，应通过与更多研究人员协商适当修改诊断标准，使其更具实用性和国际适用性。
