Stanghellini V, Cogliandro R F, De Giorgio R, Barbara G, Cremon C, Antonucci A, Fronzoni L, Cogliandro L, Naponelli V, Serra M, Corinaldesi R
Department of Clinical Medicine, University of Bologna, Bologna, Italy.
Transplant Proc. 2010 Jan-Feb;42(1):15-8. doi: 10.1016/j.transproceed.2009.12.017.
Chronic intestinal pseudo-obstruction is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time. The diagnosis is based on the evidence of typical clinical manifestations, radiological evidence of distended bowel loops with air-fluid levels, and the exclusion of any organic obstruction of the gut lumen. The radiological sign of intestinal occlusion allows the distinction from enteric dysmotility, which is characterized by better outcomes. Manometry can play a supportive role in defining the diagnosis, and differences in the manometric pattern of chronic intestinal pseudo-obstruction and enteric dysmotility have been shown. The disease is often unrecognized, and the diagnosis, therefore, delayed by several years. Thus, the majority of patients undergo useless and potentially dangerous surgeries. Long-term outcomes are generally poor despite surgical and medical therapies characterized by disabling and potentially life-threatening complications over time. A substantial percentage of patients requires parenteral nutrition. Failure of this nutritional support represents an indication for small bowel transplantation.
慢性肠道假性梗阻是一种严重且常未被识别的疾病，其特征是随着时间推移会出现致残性和潜在危及生命的并发症。诊断基于典型临床表现的证据、肠袢扩张伴气液平面的放射学证据以及排除肠腔的任何器质性梗阻。肠道闭塞的放射学征象有助于与肠道动力障碍相区分，肠道动力障碍的预后较好。测压在明确诊断方面可起辅助作用，并且已显示慢性肠道假性梗阻和肠道动力障碍在测压模式上存在差异。该疾病常未被识别，因此诊断会延迟数年。这样一来，大多数患者接受了无用且可能危险的手术。尽管手术和药物治疗随着时间推移会出现致残性和潜在危及生命的并发症，但长期预后通常较差。相当一部分患者需要肠外营养。这种营养支持失败是小肠移植的指征。
