黑斑息肉综合征一例并文献复习

发布时间：2018-09-04 16:52

【摘要】：目的：探讨黑斑息肉综合征(Peutz-Jeghers Syndrome以下简称P-J综合征或PJS)的流行病学、临床特征、影像学检查、鉴别诊断、治疗、随访等,提高PJS的诊断、治疗水平和随访意识,减少癌变的发生。方法：分析1例P-J综合征患者的临床资料,联合相关文献进行复习与总结。资料：男性青少年患者,间歇性恶心、呕吐2月余,呕吐物为黄色液体。患者口唇、指和趾端可见黑色不规则形互相不融合的色素斑,表面光滑。干预措施：行各项常规检查及胸片、腹部立位片、电子胃镜、电子结肠镜、全消化道钡剂造影以明确诊断,同时予以禁食水、胃肠减压、维持水电解质及酸碱平衡、营养支持及对症治疗,结合检查结果综合分析后予以剖腹探查、肠切除吻合术治疗,术后密切观察病情,同时出院后于我科住院随访四次。结果：胸片示：双肺纹理增重。腹部立位片示：腹部部分肠管充气,建议必要时复查。电子胃镜示：胃多发息肉。电子结肠镜示：结肠息肉。全消化道钡剂造影示：自Trietz韧带以下空肠明显扩张,考虑不完全性肠梗阻,请结合临床。术后标本肠管送病检,病检提示：(空肠)病变符合色素沉着-多发性胃肠道息肉病(Peutz-Jeghers,息肉)。结论：P-J综合征的病人多以肠梗阻或不完全性肠梗阻入院,常伴有“痛、吐、胀、闭”等临床表现。相当一部分病人有家族史,同时伴有口唇、颊粘膜、齿龈、鼻、眼周围、会阴部、指和趾端黑色不规则形互相不融合的色素斑,结合胃肠镜及病检等结果综合判断,可提高诊断的准确性。另外,此病复发率较高,注意随访。
[Abstract]:Objective: to investigate the epidemiology, clinical features, imaging examination, differential diagnosis, treatment, follow-up of Peutz-Jeghers Syndrome (P-J syndrome or PJS), and to improve the diagnosis, treatment and awareness of PJS. Reduce the incidence of cancer. Methods: the clinical data of 1 case with P-J syndrome were analyzed and reviewed and summarized. Materials: male adolescent patients, intermittent nausea, vomiting for 2 months, vomit is yellow fluid. The patient's lips, fingers and ends of the toe show black irregular color spots that are not fused with each other, and the surface is smooth. Intervention measures: routine examinations and chest films, abdominal orthotopic films, electronic gastroscopy, electronic colonoscopy, total gastrointestinal barium radiography were performed to make a definite diagnosis, while fasting water, gastrointestinal decompression, water, electrolyte and acid-base balance were maintained. Nutritional support and symptomatic treatment, combined with comprehensive analysis of the results of laparotomy, intestinal resection and anastomosis treatment, postoperative close observation of the condition, at the same time discharged from hospital in our hospital four times. Results: chest radiographs showed that the weight of both lungs was increased. Abdominal position film shows: abdominal part of the intestinal tube inflated, recommended review if necessary. Electronic gastroscopy showed multiple polyps in the stomach. Electronic colonoscopy: colonic polyps. Total gastrointestinal barium radiography showed that the jejunum dilated obviously under the Trietz ligament, considering incomplete intestinal obstruction, please combine with clinic. The pathological changes of jejunum accord with pigmentation-multiple gastrointestinal polyposis (Peutz-Jeghers, polyps). Conclusion the majority of patients with W P-J syndrome are admitted to hospital with intestinal obstruction or incomplete intestinal obstruction, often accompanied by "pain, vomiting, distension, closure" and other clinical manifestations. A considerable number of patients had a family history, accompanied by pigmented spots with irregular black irregular shapes around the eyes, perineum, fingers and toes, combined with gastroenteroscopy and pathological examination, with the results of lip, buccal mucosa, gingival, nose, eye, perineum, finger and toe, and so on. It can improve the accuracy of diagnosis. In addition, the recurrence rate of this disease is high, pay attention to follow-up.
【学位授予单位】：兰州大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R596.1

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