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目的探讨黑斑息肉综合征(Peutz-Jegherssyndrome,PJS)的诊断方法和合理的治疗方案。方法对我院收治的6例PJS患者的临床资料进行分析。结果6例均以反复发作的腹痛起病,以肠套叠和息肉出血入院。4例患者的父亲,3例患者的祖父和1例患者的母亲患有PJS,3例患者有癌症家族史。病例4和病例5由于肠道内息肉复发引起肠套叠或反复腹痛而多次接收手术治疗。病例1和4的息肉同时伴有腺瘤样改变,病例2息肉同时伴有胃癌。治疗主要为息肉切除和部分小肠、结肠切除。结论PJS患者有明显的癌症家族史和息肉的高复发率;手术是治疗的主要手段;对PJS患者的胃肠道和其他有可能恶变的脏器应进行随访。
Objective To investigate the diagnosis and rational treatment of Peutz-Jeghers syndrome (PJS). Methods The clinical data of 6 PJS patients admitted to our hospital were analyzed. Results 6 cases were recurrent abdominal pain onset, intussusception and polyp bleeding. The father of 4 patients, the grandfather of 3 patients and the mother of 1 patient had PJS, and 3 patients had a family history of cancer. Case 4 and Case 5 received multiple surgeries for intussusception or recurrent abdominal pain due to recurrent polyps in the gut. Case 1 and 4 polyps accompanied by adenomatous changes, Case 2 polyps accompanied by gastric cancer. The main treatment for polyp resection and part of the small intestine, colon resection. Conclusions PJS patients have obvious family history of cancer and high recurrence rate of polyps. Surgery is the main method of treatment. Gastrointestinal tract and other malignant organs in PJS patients should be followed up.