内脏肌病致急性假性结肠梗阻

Visceral myopathy causing acute colonic pseudoobstruction

内脏肌病是一种由内脏平滑肌萎缩、缺失和纤维化引起的罕见疾病。病变常累及消化道，临床表现随病变部位和程度不同而异。小肠病变可表现为腹胀、腹泻及呕吐等消化不良症状，累及结肠则表现为急性假性肠梗阻。本病可继发营养不良、低蛋白血症等。常与麻痹性肠梗阻，慢性便秘，进行性全身硬化等疾病相混淆，诊断上存在一定困难。本病病程进展通常较缓慢，远期预后不良。本文回顾性分析吉林大学白求恩第一医院收治的l例内脏肌病致急性假性结肠梗阻患者的临床资料，总结该疾病的诊断和治疗经验。

Visceral myopathy is a rare disorder characterized by atrophy, losing and fibrosis of visceral smooth muscle. Digestive tract is often invaded by the lesions, and the symptoms are various according to different lesions and degrees. Small intestinal involvement is characterized by abdominal distension, diarrhea and vomiting. Colon involvement is characterized by chronic intestinal pseudoobstruction. Malnutrition and hypoproteinemia may be secondary to this disease. The diagnosis of visceral myopathy is difficult, paralytic ileus, chronic constipation and systemic sclerosis must be considered in the differential diagnosis. The progression of the disease is slow, and the long- term prognosis is poor. In this article, the diagnosis and treatment of visceral myopathy causing acute intestinal pseudoobstruction were introduced based on the clinical data of 1 patient.