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What is chronic intestinal pseudo-obstruction?

慢性肠pseudo-obstruction (CIP)是一个rare disorder in which intestinal nerve or muscle problems prevent food, fluid, and air from moving through the stomach and intestines. The child experiences the symptoms of an intestinal blockage, though no actual physical blockage exists. Over time, children with CIP can become malnourished because their gastrointestinal tracts are unable to absorb food and get nutrition.

每年在美国,大约有100名儿童出生于先天性CIP。CIP是可以通过适当的医疗服务来管理的终身状况。

What are the symptoms of chronic intestinal pseudo-obstruction?

慢性肠道伪腹结构可防止液体和食物通过胃和肠道移动。症状各不相同,具体取决于肠道的哪一部分受到影响。常见症状包括:

  • 腹部扩张(腹部肿胀或肿胀)
  • 呕吐
  • 便秘
  • abdominal pain
  • diarrhea
  • feeling full after a small snack
  • 食物厌恶
  • 与延伸或阻塞性症状有关的疼痛

缺乏肠功能会导致并发症。通常可以防止人体吸收营养。结果,许多孩子患有营养不良,failure to thrive, and weight loss. It is common for doctors to suspect and diagnose this condition soon after birth or before a child’s first birthday in the cases of primary pseudo-obstruction.

是什么原因导致慢性肠道伪腹结构?

慢性肠道伪腹结构是由神经或肌肉问题引起的,这些问题阻止了肠道正常收缩以通过肠道移动食物,液体和空气。在许多情况下,根本原因是未知的。在儿童中,这种病通常是先天性的,这意味着大多数患有疾病的孩子是天生的。

慢性肠道伪腹结构可能是由神经或胃中的肌肉的问题引起的:

  • 神经疗法:when the stomach and intestines contract, but the contractions are unsynchronized due to a problem with the nerves in the gastrointestinal tract
  • myogenic:when the stomach does not contract or the contractions are weak due to a muscular problem

有时,肠道运动的问题可能是其他潜在疾病(例如代谢问题,线粒体疾病和手术)继发的。这些问题被认为是伪攻击的次要原因。他们可能与具有主要形式的儿童遇到相同的问题。

我们如何照顾慢性肠道伪腹结构

Motility and Functional Gastrointestinal Disorders Centerat Boston Children’s Hospital provides access to state-of-the-art specialized multi-disciplinary gastroenterology services including the most advanced testing, new therapy, and access to more options for your child’s treatment plan. Our expert team will work together and with your family to develop an individualized treatment plan that addresses the full spectrum of your child’s health needs and provides the best outcomes and quality of life.

慢性肠道伪造|Diagnosis & Treatments

如何诊断慢性肠道伪腹结构?

因为慢性肠道伪腹结构(CIP)的症状类似于其他gastrointestinal conditions, your child's doctor may need to order several tests before making a formal diagnosis.

Your child's doctor may order one or more of the following tests and procedures:

  • 成像研究, 如x-rays, can rule out an intestinal obstruction.
  • anmeasure pressure in the bowel and can help diagnose CIP without more invasive testing. During anantroduodenal manometry测试,医生通过鼻子或现有的胃造口术(进食管)喂入胃和小肠以测量压力。对于结肠测压,医生做同样的事情,但通过结肠将管子喂入肠道。
  • Laparotomyis a surgical procedure that is rarely necessary. A surgeon makes an abdominal incision so they can examine and biopsy the intestines.

如何处理肠道伪造?

虽然尚无伪造的已知治疗方法,但适当的治疗可以稳定病情,防止并发症并改善您的孩子的生活质量,因此他们可以参加典型的儿童活动。

Treatment may include:

  • Nutritional support therapy适当的营养是CIP儿童的重中之重。CIP的孩子应该在整天吃几顿小餐,易于消化的食物。甚至无法吃小饭的孩子可能需要肠内营养,该营养可通过喂食管将液体饮食供应到胃或肠道。在更严重的情况下,孩子的医生可能会建议肠胃外营养,通过导管直接向静脉提供营养。
  • Surgical decompression:为了减轻肠中的压力,外科医生可以将喂食管直接插入胃中,或在腹部形成开口,肠道可以通过该开口释放气体和空凳子。
  • Medications:Prokinetic drugs may improve motility, the ability to move food through the intestines, in some children by aiding contractions. Depending on the child's symptoms, the child's clinician may also prescribe antibiotics, anti-nausea medications, anti-diarrheal medications, or laxatives.
  • 疼痛管理有几种选择可以控制和缓解CIP相关的疼痛和不适,包括口服药物,甚至硬膜外麻醉。但是,止痛药也可以减慢消化,并应为CIP儿童提供少量使用。
  • 小肠移植在严重的情况下,可能需要用intestinal and multivisceral transplant

慢性肠道伪造|Programs & Services

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