CAJ | 학술논문

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1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.
내장기병시일충유내장평활기위축、결실화섬유화인기적한견질병.병변상루급소화도,림상표현수병변부위화정도불동이이.소장병변가표현위복창、복사급구토등소화불량증상,루급결장칙표현위급성가성장경조.본병가계발영양불량、저단백혈증등.상여마비성장경조,만성편비,진행성전신경화등질병상혼효,진단상존재일정곤난.본병병정진전통상교완만,원기예후불량.본문회고성분석길림대학백구은제일의원수치적1례내장기병치급성가성결장경조환자적림상자료,총결해질병적진단화치료경험.
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 longterm 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.