儿童色素沉着息肉综合征及文献复习

目的:对3例以腹痛、贫血、便血、肠梗阻为首诊的儿童少见疾病进行确诊。方法:根据患儿家族史、临床观察、肠镜、X线检查、病理检查、手术确诊。结果:儿童色素沉着息肉综合征常以贫血、腹痛、便血、肠梗阻等为主要表现。可有遗传性,可散发。常伴恶性病变。结论:应提高对儿童色素沉着息肉综合征的认识,以利早期诊断,恰当治疗、随访。

PEUTZ- JEHGERS SYNDROME IN CHILDREN AND THE REVIEW OF LITERATURE

Objective:To make a definit diagnosis for three children with Peutz-Jeghers syndrome who were first diagnosed to be bellyache,anemia,ileus,and bleed in stool.Methods: Peutz-Jeghers syndrome was diagnosed based on the familial history,clinic symptoms,X-ray examination,pathology and operation.Results:Bellyache,anemia,ileus,and bleed in stool were the most common symptoms of Peutz-Jeghers of children caused by gastrointestinal polyposis and disorders.Half of the patients had familial history.Most of them would complicated with cancer in their whole life.Conclusion:It is important to diagnose Peutz-Jehgers Syndrome early,and also the disease should be treated early and properly.