关键词: ACTG2 mutations anti-Hu antibodies pseudo-obstruction visceral myopathy visceral neuropathy

Mesh : Adult Humans Intestinal Pseudo-Obstruction / diagnosis etiology therapy Intestinal Obstruction / complications Prognosis Chronic Disease Syndrome

来  源:   DOI:10.1111/nmo.14715

Abstract:
Chronic intestinal pseudo-obstruction is a rare and heterogeneous syndrome characterized by recurrent symptoms of intestinal obstruction with radiological features of dilated small or large intestine with air/fluid levels in the absence of any mechanical occlusive lesion. Several diseases may be associated with chronic intestinal pseudo-obstruction and in these cases, the prognosis and treatment are related to the underlying disease. Also, in its \"primary or idiopathic\" form, two subgroups of patients should be determined as they require a more specific therapeutic approach: patients whose chronic intestinal pseudo-obstruction is due to sporadic autoimmune/inflammatory mechanisms and patients whose neuromuscular changes are genetically determined. In a context of a widely heterogeneous adult population presenting chronic intestinal pseudo-obstruction, this review aims to summarize a practical diagnostic workup for identifying definite subgroups of patients who might benefit from more specific treatments, based on the etiology of their underlying condition.
摘要:
慢性肠假性梗阻是一种罕见且异质的综合征,其特征是肠梗阻的反复发作症状,具有小肠或大肠扩张的放射学特征,在没有任何机械性闭塞性病变的情况下具有空气/液体水平。几种疾病可能与慢性肠道假性梗阻有关,在这些病例中,预后和治疗与基础疾病有关。此外,以其“原发性或特发性”形式,应确定两个亚组患者,因为它们需要更具体的治疗方法:由散发性自身免疫/炎症机制引起的慢性假性肠梗阻患者和由基因决定神经肌肉改变的患者.在广泛异质的成年人群表现出慢性假性肠梗阻的背景下,这篇综述旨在总结一个实用的诊断工作,以确定可能从更具体的治疗中受益的患者亚组,基于他们潜在病情的病因。
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