Objective To analyse the clinical features of Peutz-Jeghers syndrome and its canceration, and to summarize the management and the follow-up strategy for patients with Peutz-Jeghers syndrome. Methods Clinical data of 30 patients with Peutz-Jeghers syndrome dating from October 1985 to September 2005 in West China Hospital of Sichuan University were analyzed retrospectively. Results Fourteen (46.67%) definite family histories of Peutz-Jeghers syndrome were found. Pigmentation of skin and mucosa, abdominalgia and hematochezia were major clinical manifestations of the syndrome. There were 18 patients (60.00%) complicated with intussusception and acute intestinal obstructions, 16 patients (53.33%) with gastrointestinal bleeding, and 6 patients (20.00%) developed malignancy. The frequent types of malignancy were carcinoma of small intestine (3 cases), colon carcinoma (2 cases) and gastric carcinoma (1 case) in order. The mean age of the canceration-diagnosed patients was 32 years old. The type of pathohistology of all the malignancy was poorly differentiated mucus adenocarcinoma. High-frequency endoscopic electroresection, orthdox polypectomy and enterectomy were the major means of treatment. Conclusion Patients with Peutz-Jeghers syndrome are at high risk of canceration at relatively early ages and usually the differentiation of the tumor is poor. Endoscopy should be performed regularly and the high-frequency electroresection is an effective therapy in disposing intestinal polyp. Screening can also improve the efficacy of Peutz-Jeghers syndrome.
目的 分析Peutz-Jeghers综合征临床特点，以提高诊治水平。 方法 2008年11月-2010年1月对8例Peutz-Jeghers综合征的临床资料进行回顾性分析。 结果 男女各4例，年龄13～41岁，病程20 d～40年。8例均有黑斑和息肉，5例有明确的家族史。首发表现以腹痛为主，轻度抑郁表现2例，并发肠套叠2例，结肠癌1例。病理报告：错构瘤4例。外科手术治疗1例，结肠镜治疗6例，保守治疗1例。 结论 Peutz-Jeghers综合征易并发抑郁症、肠套叠和恶性肿瘤，患者需心理治疗和遗传咨询，肠镜下息肉切除是主要手段。
Objective To investigate the medication advancement of gastrointestinal polyposis in patients with Peutz-Jeghers syndrome (PJS). Methods Literatures about the medication advancement on gastrointestinal polyposis of PJS were reviewed and analyzed. The recent development of targeting drugs, especially the data of cyclooxygenase-2 selective inhibitors and rapamycin, were emphatically summarized. Results With the deep investigation of PJS and application of selective drugs, the medication of gastrointestinal polyposis in cases of PJS has got more advancement. The extensive use of synthetic cyclooxygenase-2 inhibitors and rapamycin in clinic developed a new way to treat gastrointestinal polyposis of PJS. Conclusion The cyclooxygenase-2 selective inhibitors and rapamycin have the following features: noninvasive, high selectivity and good curative effects. They have splendid prospects in the clinical treatment of gastrointestinal polyposis in patients with PJS and are bring the treatment of gastrointestinal polyposis in cases of PJS into a targeting therapy phase.
Objective To investigate the clinicopathological features and clinical subtypes of Peutz-Jeghers syndrome (PJS) in Chinese cases. Methods The clinical and pathological data of 295 patients with PJS who were treated in Air Force General Hospital from Nov. 1994 to Aug. 2017 were retrospectively analyzed and a multifactor statistical study was carried out on. Results Two hundreds and ninety-five patients with PJS belonged to 7 nationalities and came from 26 provinces and urban areas. 99.0% (292/295) of the patients had black spots on the lip and buccal mucosa, and the median occurrence time was 2 years old (0–33 years). The median age of inital diagnosis and treatment was 15 years old (1–45 years). The median interval time between the occurrence of black spots and abdominal symptoms was about 10 years (0–45 years). PJS hamartoma polyps were found in alimentary canals of 293 patients (99.3%), and 96.9% distributed in the duodenum and small intestine (n=284), 90.4% distributed in the colorectal (n=265), 79.9% distributed in the stomach (n=234). Patients of black spot appearing at age <3 years and (or) initial treatment at age <14 years were classified as early-onset subtype, otherwise they could be included in delayed-onset subtype. Conclusions The clinical features of PJS are prominent and the harm of PJS hamartoma polyps is serious. The black spots on the lip and buccal mucosa can be used as an early warning signal to divide the PJS patients into 2 clinical subtypes, which should be differentiated in clinical therapy and follow-up strategy.