Jain Dhanpat, Moussa Khalid, Tandon Manish, Culpepper-Morgan Joan, Proctor Deborah D
Department of Anatomic Pathology, Program in Gastrointestinal Pathology, Yale University School of Medicine, New Haven, Connecticut, USA.
Am J Gastroenterol. 2003 Mar;98(3):618-24. doi: 10.1111/j.1572-0241.2003.07295.x.
Idiopathic intestinal pseudo-obstruction is characterized by the failure of the intestinal tract to propel its contents appropriately. This leads to signs and symptoms of bowel obstruction and, in the absence of an associated systemic disorder or the administration of drugs known to result in bowel dysmotility, is termed chronic idiopathic intestinal pseudo-obstruction (CIIP). Histopathologically, patients with CIIP can be characterized as having either myopathic or neuropathic forms, but the large majority of patients do not show any specific histological changes. Interstitial cells of Cajal (ICC) have been shown to be the pacemaker cells of the bowel and have been implicated in the pathogenesis of CIIP. The aim of this study was to compare the number and distribution patterns of c-kit+ ICC in CIIP in patients with mechanical bowel obstruction, other bowel motility disorders, and normal controls.
Six patients with CIIP, six age-matched normal controls, nine patients with mechanical bowel obstruction, and 18 patients with other motility disorders (non-CIIP), including 10 with secondary intestinal pseudo-obstruction, were studied. Toluidine blue, Masson's trichrome, and S-100 immunostaining were performed in all subjects. The ICC were identified by an indirect immunoperoxidase method using a polyclonal c-kit antibody.
All six patients with CIIP showed total absence of c-kit+ ICC. A subject with neonatal meconium ileus in the non-CIIP group showed patchy areas devoid of c-kit+ ICC amid normal areas. The c-kit+ ICC had a normal number and distribution pattern in all patients with mechanical obstruction and in the remaining 17 non-CIIP subjects.
It seems that CIIP is characterized by a total loss of c-kit+ ICC. ICC may play an important role in the etiopathogenesis of CIIP and transient neonatal meconium syndrome, and staining for c-kit receptor may be very useful in the evaluation of motility disorders of the bowel.
特发性肠道假性梗阻的特征是肠道无法正常推进其内容物。这会导致肠梗阻的体征和症状，并且在没有相关全身性疾病或未使用已知会导致肠道运动障碍的药物的情况下，被称为慢性特发性肠道假性梗阻（CIIP）。在组织病理学上，CIIP患者可分为肌病型或神经病变型，但绝大多数患者没有任何特定的组织学变化。 Cajal间质细胞（ICC）已被证明是肠道的起搏细胞，并与CIIP的发病机制有关。本研究的目的是比较CIIP患者、机械性肠梗阻患者、其他肠道运动障碍患者和正常对照者中c-kit + ICC的数量和分布模式。
研究了6例CIIP患者、6例年龄匹配的正常对照者、9例机械性肠梗阻患者和18例其他运动障碍（非CIIP）患者，其中包括10例继发性肠道假性梗阻患者。对所有受试者进行甲苯胺蓝、Masson三色染色和S-100免疫染色。使用多克隆c-kit抗体通过间接免疫过氧化物酶法鉴定ICC。
所有6例CIIP患者均显示c-kit + ICC完全缺失。非CIIP组中一名患有新生儿胎粪性肠梗阻的受试者在正常区域中显示出c-kit + ICC缺失的片状区域。在所有机械性梗阻患者和其余17例非CIIP受试者中，c-kit + ICC的数量和分布模式正常。
似乎CIIP的特征是c-kit + ICC完全丧失。 ICC可能在CIIP和短暂性新生儿胎粪综合征的病因发病机制中起重要作用，并且c-kit受体染色在评估肠道运动障碍中可能非常有用。
