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贲门失弛缓症

Achalasia.

作者信息

Dughera L, Cassolino P, Cisarò F, Chiaverina M

机构信息

Motility and Endoscopy Unit, Department of Internal Medicine, San Giovanni Battista Hospital, Turin, Italy.

出版信息

Minerva Gastroenterol Dietol. 2008 Sep;54(3):277-85.

Abstract

Achalasia is a rare motor disorder of the esophagus, characterized by the absence of peristalsis and impaired swallow-induced relaxation. These motor abnormalities result in stasis of ingested food in the esophagus, leading to clinical symptoms, such as dysphagia, regurgitation of food, retrosternal pain and weight loss. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. In terms of diagnosis, esophageal manometry is the gold standard to diagnose achalasia. Still, its role in post-treatment surveillance remains controversial. Radiological studies support the initial diagnosis of achalasia and have been proposed for detecting preclinical symptomatic recurrence. Although endoscopy is considered to have a poor sensitivity and specificity in the diagnosis of achalasia, it has an important role in ruling out secondary causes of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the lower esophageal sphincter (LES) pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids.

摘要

贲门失弛缓症是一种罕见的食管运动障碍性疾病,其特征为缺乏蠕动以及吞咽诱发的松弛受损。这些运动异常导致摄入的食物在食管内淤滞,从而引发诸如吞咽困难、食物反流、胸骨后疼痛和体重减轻等临床症状。病因不明。虽然有一些家族性病例的报道,但家族性发病的罕见性并不支持遗传是一个重要病因的假说。有报道称贲门失弛缓症与病毒感染以及针对肌间神经丛的自身抗体有关,但因果关系仍不明确。在诊断方面,食管测压是诊断贲门失弛缓症的金标准。不过,其在治疗后监测中的作用仍存在争议。放射学研究有助于贲门失弛缓症的初步诊断,并已被用于检测临床前症状复发。虽然内镜检查在贲门失弛缓症诊断中的敏感性和特异性被认为较差,但它在排除贲门失弛缓症的继发原因方面具有重要作用。治疗主要是姑息性的。目前的内科和外科治疗选择(气囊扩张、手术肌切开术和药物)旨在降低食管下括约肌(LES)压力,并通过潴留食物和液体的重力及静水压力促进食管排空。

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