事件记录
面试官: Hirschsprung's disease is a rare intestinal disorder that can severely impact a child's ability to pass stool. What treatments are available and what you can expect for your child on 范围.
播音员: 由研究支持的专家大发娱乐提供的健康信息. 这是来自犹他大学健康中心的scoperadio.com.
面试官: 我和迈克尔·罗林斯在一起, a professor of surgery in the Division of Pediatric Surgery at the University of Utah Medical School and Primary Children's Hospital, 大发娱乐要讨论的是先天性巨结肠病. 我的发音对吗，巨结肠?
Dr. 罗林斯: 对，没错.
面试官: 那么这种疾病到底是什么呢?
Dr. 罗林斯: Hirschsprung disease is a disorder where the nerve cells of the rectum and/or part of the colon don't form normally, and these nerve cells are responsible for relaxation of the rectum and colon to allow the passage of stool. So basically, a child is born with an intestinal obstruction because they're unable to pass stool.
面试官: 所以我认为这可能会很严重. 这种疾病有多常见?
Dr. 罗林斯: It occurs in about 1 in 5,000 live births, and yes, it is serious. It's a problem that most of the time will progress to severe 腹胀 or 呕吐, 喂养不耐受, and can even progress to a life-threatening infection called enterocolitis or an intestinal perforation if it's not recognized within the first few days of life.
面试官: What are some of the ways that physicians, surgeons kind of treat this disorder?
Dr. 罗林斯: 大多数婴儿会排出胎便, 第一个凳子, 在出生后的两天内, 高达98%左右. So probably the most common way that this is diagnosed is the baby is born and they don't pass stool in the first two days of life. Second most common way would be that the baby doesn't tolerate feeds in the first one to two days of life, 变得明显膨胀, 腹部膨胀, 或者他们在呕吐, 也可能不排便或排便很少.
Those are red flags and those prompt further either consultation to a pediatric surgical service or further investigation, which most commonly the first study would be plain abdominal x-ray or what's called a contrast enema, where a small tube is placed in the child's rectum and contrast fluid, 注入造影剂来观察直肠和结肠.
面试官: 然后你如何治疗或纠正? 你对这种障碍做了什么干预?
Dr. 罗林斯: 巨先天性疾病需要手术矫正. So as I mentioned a little earlier, it's a spectrum of a disease. 所以大肠的末端可能会受到影响, 所以只有直肠可能会受到影响, 但是上游的结肠长度是可变的, or even in more or the most extreme cases even part of the small intestine may be affected, 所以大便通不了. So the treatment options would be different really based on the level of the disease.
80%只影响直肠，也就是大发娱乐所说的短段. And in those babies, the treatment option, they can be treated in three different ways. The first thing is a rectal biopsy is performed at the bedside to make a definitive diagnosis. So the pathologist won't see the nerve cells when they look at it under the microscope. 所以一旦你有了明确的诊断, then you can either proceed to the operating room shortly thereafter and determine the level of the normal intestine, 切除或取出异常部分, 把正常的肠道缩小到一个小的, 肛管上方的小直肠袖. 这是一次手术.
Another treatment method may be to perform daily irrigations or washout of the rectum and colon. 父母经常在家里这样做. 一旦孩子有机会成长, 变大一点, we'll come back around three months of age or so and do that one-stage operation at that point. 有时, 这在技术上更简单, 然后, 你知道, the babies had a chance to go home and be with parents and develop that normal relationship.
And then the third way to treat it would be with a colostomy at birth. 所以去手术室吧, determine the level of the normal intestine and bring that up to the abdominal wall so that stool empties into a bag. 一旦孩子长大了，长大了, 回去做最后的手术, where the abnormal portion is resected and the normal portion is brought down to the bottom.
面试官: And what kind of care should a parent expect after this procedure?
Dr. 罗林斯: So the tricky part of Hirschsprung's disease is intuitively you'd think that, "Okay, 不正常的肠道消失了, 我的孩子痊愈了.“大发娱乐想了很长时间. 但大发娱乐已经开始意识到，大发娱乐作为一个外科社区, medical community have begun to realize that that's not actually the case.
你知道, 在过去, 大发娱乐会做这个手术, 几个星期后，大发娱乐再去看孩子, 一切看起来都很棒, 伟大的行动, 你知道, 准备好了. And those children would develop problems with infections of the intestine called enterocolitis, 会不会得细菌过度生长导致严重感染, 哪些症状是发烧, 腹胀, 呕吐, 严重爆发性腹泻. 治疗方法是冲洗, 直肠褪色, 哪些父母可以在家里做，哪些在大发娱乐做, 在大学医院的小学儿童医院, we teach all of our families how to do that so that if those symptoms develop, 他们可以大发娱乐, 让大发娱乐知道, 然后在家里开始治疗.
这是短期的, 你知道, what we worry about after we're satisfied with the surgical side healing and 诸如此类. 所以大发娱乐一开始担心的是小肠结肠炎. Seems like that becomes less of a problem after the first couple of years of life.
这个问题似乎在儿童时期最常见, 甚至到了青春期, 是便秘. And it's constipation beyond what you think of when you think of constipation, maybe hard stool that's infrequent that the pediatrician or primary care provider treats routinely each day. This 是便秘 that requires often a formal bowel management program with a high-dose stimulant laxative therapy to get the large intestine to squeeze and empty normally. 如果不承认这种便秘, 不及时治疗, 随着时间的推移, 它会导致结肠功能失调. 所以大发娱乐向家庭强调, 你知道, this is a long-term relationship with us as the care team in order to avoid these complications.
面试官: 有了这种长期护理, what kind of quality of life can a parent expect for their child with this disorder?
Dr. 罗林斯: The great news is, as I mentioned, our thought process 在过去 was not complete. Now, 大发娱乐正在认识到这些问题, 通过早期管理, 这些孩子会很有能力, 你知道, 做运动的正常儿童, 去普通学校, 在外过夜, camps, 诸如此类, they just may have to take medication each day in the form of a laxative, 你们都知道, I mean, I explain to children and to their families that you wouldn't think anything about taking your inhaler every day if you had asthma. 你们知道，这是一样的.
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