成人慢性特发性肠假性梗阻的自然病史：一项单中心研究。

Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study.

作者信息

Stanghellini Vincenzo, Cogliandro Rosanna F, De Giorgio Roberto, Barbara Giovanni, Morselli-Labate Antonio M, Cogliandro Laura, Corinaldesi Roberto

机构信息

Department of Internal Medicine and Gastroenterology, University of Bologna, Italy.

出版信息

Clin Gastroenterol Hepatol. 2005 May;3(5):449-58. doi: 10.1016/s1542-3565(04)00675-5.

DOI:10.1016/s1542-3565(04)00675-5

PMID:15880314

Abstract

BACKGROUND & AIMS: Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Intestinal motor abnormalities have long been identified in CIIP patients. Little is known of the natural history of the disease in adults. This study evaluated the clinical course of CIIP over time.

METHODS

Fifty-nine consecutive CIIP patients without underlying collagen, vascular diseases, or mitochondrial cytopathies were evaluated between 1985 and 2001. Family history, onset of digestive symptoms, previous surgeries, episodes suggestive of subacute intestinal obstruction, digestive symptoms, body mass index, and feeding habits were recorded. Small bowel manometry was performed by a perfusion technique, and abnormal motor patterns were visually identified. Full-thickness biopsies were available in 11 cases and were processed for immunohistochemical analysis of myogenic and neurogenic components of the gut wall.

RESULTS

Patients were prospectively followed up for a median of 4.6 years (range, 1-13 years). Diagnosis was often made several years after symptom onset (median, 8 years). Thus, the majority of patients (88%) underwent useless and potentially dangerous surgeries (mean, 2.96 per patient). Manometry invariably showed abnormal motor patterns. Pathologic findings included neuropathies in all investigated cases and abnormalities of interstitial cells of Cajal in 5 of 11 cases. Long-term outcome was generally poor despite surgical and medical therapies; 4 patients died of disease-related complications, 4 underwent small bowel transplantation, almost one third required long-term home parenteral nutrition, and two thirds had some sort of nutritional limitations.

CONCLUSIONS

CIIP is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time.

摘要

背景与目的

慢性特发性肠假性梗阻（CIIP）是一种罕见疾病，其特征为在无器质性、全身性或代谢性疾病的情况下出现类似机械性梗阻的发作。CIIP患者长期以来一直被发现存在肠道运动异常。对于该疾病在成人中的自然病程了解甚少。本研究评估了CIIP随时间的临床病程。

方法

对1985年至2001年间连续纳入的59例无潜在胶原血管疾病或线粒体细胞病变的CIIP患者进行评估。记录家族史、消化症状发作情况、既往手术史、提示亚急性肠梗阻的发作情况、消化症状、体重指数和饮食习惯等信息。采用灌注技术进行小肠测压，并通过肉眼识别异常的运动模式。11例患者获取了全层活检组织，并对肠壁肌源性和神经源性成分进行免疫组化分析。

结果

对患者进行前瞻性随访，中位随访时间为4.6年（范围1 - 13年）。诊断通常在症状出现数年之后（中位时间为8年）。因此，大多数患者（88%）接受了无效且可能危险的手术（平均每位患者2.96次）。测压始终显示异常的运动模式。病理结果包括所有研究病例均存在神经病变，11例中有5例存在Cajal间质细胞异常。尽管接受了手术和药物治疗，但长期预后通常较差；4例患者死于疾病相关并发症；4例接受了小肠移植；近三分之一的患者需要长期家庭肠外营养；三分之二的患者存在某种营养限制。