摘要
目的总结分析儿童黑斑息肉综合征(PJS)的临床特点。方法回顾性分析2009年1月至2017年6月郑州大学第一附属医院收治的14例黑斑息肉综合征患儿的临床资料,结合相关文献数据,分析儿童PJS的发病、诊断及治疗特点。结果 14例患儿发病年龄2~17岁,均出现口唇及四肢末端色素沉积,8例有家族遗传史,11例患儿有肠梗阻、肠套叠及便血等消化道症状;9例手术辅以内镜切除、灼烧等治疗,1例单纯手术切除息肉治疗,4例患儿仅单纯行内镜下切除息肉治疗,其中1例手术患儿切除2/3长度小肠,确诊为短肠综合征;息肉遍布胃肠道,以小肠为主,息肉病理主要为错构瘤样息肉,无恶变;随访14例患儿胃及结肠息肉复发率为100%,均定期接受内镜下切除,0例患儿因再次出现小肠息肉行外科手术治疗。结论儿童黑斑息肉综合征各年龄层均可发病,有家族遗传倾向,以消化道症状多见,胃及结肠息肉可经内镜下切除,小肠息肉引起的肠套叠、肠梗阻等需要外科手术治疗,患儿需定期复查及随访。
Objective To summarize and analyze the clinical features of children＇ s Peutz - Jeghers syndrome. Method The clinical data of fourteen children with Peutz - Jeghers syndrome who were admitted to the First Affiliated Hospital of Zheng- zhou University from January of 2009 to June of 2017 were retrospectively analyzed. The incidence, diagnosis and treatment char- acteristics of Peutz - Jeghers syndrome in children were analyzed following relative literatures. Results The onset age of 14 chil- eren was from 2 to 17 years old, and pigmentation occurred on the lips and extremities of all chileren. There were 8 children who had a family history of PJS, 11 children had digestive tract symptoms such as ileus, indigitation and hematochezia, ect. Among the 14 children, nine children were cured with operation under endoscopic resection and coagulation, one child was cured with operation alone, and four children were cured with endoscopic resection alone. One of the children who were cured with operation was diagnosed with short bowel syndrome as 2/3 length of the small intestine was taken during the operation. Polyps were ob- served throughout the gastrointestinal tract, and most of the polyps were located in the small intestine. Hamartoma polyp was the main pathology type, and no malignant transformation was found in this survey. Recurrence rate of the polyps which were located in the stomach and colon was 100%, and all the children with recurrence were treated with endoscopic resection regularly with no one treated with reoperation. Conclusion Peutz - Jeghers syndrome occured in children of all ages, and it has the characteristic of familial and the obvious tendency of heredity. Children with PJS manifested digestive symptoms. Gastric and colon polyps can be removed with endoscopic resection, and ileus and indigitation which were caused by PJS should be cured with operation. All the children should be followed up and given re -diagnose at regular intervals.
出处
《河南医学研究》
CAS
2017年第23期4237-4239,共3页
Henan Medical Research
关键词
黑斑息肉综合征
儿童
临床特点
Peutz - Jeghers syndrome
children
clinical features
