【摘要】：目的：探討黑斑息肉綜合征(Peutz-Jeghers Syndrome以下簡稱P-J綜合征或PJS)的流行病學(xué)、臨床特征、影像學(xué)檢查、鑒別診斷、治療、隨訪等,提高PJS的診斷、治療水平和隨訪意識,減少癌變的發(fā)生。方法：分析1例P-J綜合征患者的臨床資料,聯(lián)合相關(guān)文獻進行復(fù)習(xí)與總結(jié)。資料：男性青少年患者,間歇性惡心、嘔吐2月余,嘔吐物為黃色液體。患者口唇、指和趾端可見黑色不規(guī)則形互相不融合的色素斑,表面光滑。干預(yù)措施：行各項常規(guī)檢查及胸片、腹部立位片、電子胃鏡、電子結(jié)腸鏡、全消化道鋇劑造影以明確診斷,同時予以禁食水、胃腸減壓、維持水電解質(zhì)及酸堿平衡、營養(yǎng)支持及對癥治療,結(jié)合檢查結(jié)果綜合分析后予以剖腹探查、腸切除吻合術(shù)治療,術(shù)后密切觀察病情,同時出院后于我科住院隨訪四次。結(jié)果：胸片示：雙肺紋理增重。腹部立位片示：腹部部分腸管充氣,建議必要時復(fù)查。電子胃鏡示：胃多發(fā)息肉。電子結(jié)腸鏡示：結(jié)腸息肉。全消化道鋇劑造影示：自Trietz韌帶以下空腸明顯擴張,考慮不完全性腸梗阻,請結(jié)合臨床。術(shù)后標(biāo)本腸管送病檢,病檢提示：(空腸)病變符合色素沉著-多發(fā)性胃腸道息肉病(Peutz-Jeghers,息肉)。結(jié)論：P-J綜合征的病人多以腸梗阻或不完全性腸梗阻入院,常伴有“痛、吐、脹、閉”等臨床表現(xiàn)。相當(dāng)一部分病人有家族史,同時伴有口唇、頰粘膜、齒齦、鼻、眼周圍、會陰部、指和趾端黑色不規(guī)則形互相不融合的色素斑,結(jié)合胃腸鏡及病檢等結(jié)果綜合判斷,可提高診斷的準(zhǔn)確性。另外,此病復(fù)發(fā)率較高,注意隨訪。
[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.
【學(xué)位授予單位】：蘭州大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2015
【分類號】：R596.1

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