PEUTZ-JEGHERS SYNDROME - A CLINICOPATHOLOGIC SURVEY OF THE HARRISBURG FAMILY WITH A 49-YEAR FOLLOW-UP
PEUTZ-JEGHERS SYNDROME - A CLINICOPATHOLOGIC SURVEY OF THE HARRISBURG FAMILY WITH A 49-YEAR FOLLOW-UP
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DOI：
10.1016/s0016-5085(88)80074-x
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发表时间：
1988-12-01
期刊：
影响因子：
29.4
通讯作者：
ABT, AB
中科院分区：
文献类型：
--
作者：
FOLEY, TR;MCGARRITY, TJ;ABT, AB
在Jeghers报道的原始Peutz-Jeghers家族中，“哈里斯堡家族”现在已经被跟踪了49年。他们的12名受影响的家庭成员组成了报道的最大的Peultz-Jeghers亲属。这个家族的病程说明黑斑息肉综合征不是一种良性疾病。一个家族成员在错构瘤伴腺瘤样改变中发展为十二指肠癌;这种十二指肠进展以前没有报道。10例患者接受了75例息肉切除术。1例患者出现短肠综合征。3名患者在年轻时死亡。12例受影响患者中有2例发生胃肠道恶性肿瘤，提示Peutz-Jeghers综合征可能是一种癌前病变。因此，即使是无症状的胃、十二指肠和结肠息肉也应该通过内窥镜切除。如果需要手术干预，术中内镜下息肉切除术可预防短肠综合征的发生。对患者及其家属进行结肠镜筛查可能是有益的，对肠外恶性肿瘤的监测似乎是必要的。
Of the original Peutz-Jeghers families reported by Jeghers, the "Harrisburg Familiy" has now been followed for 49 yr. Their 12 affected family members comprise the largest Peultz-Jeghers kindred reported. The course of this family illustrates that Peutz-Jeghers syndrome is not a benign disease. One family member developed a duodenal carcinoma in a hamartoma with adenomatous changes; this progression in the duodenum has not previously been reported. Ten patients underwent 75 polypectomies. One patient developed short bowel syndrome. Three patients died in young adulthood. The developmental of gastrointestinal malignancy in 2 of 12 affected patients suggests that Peutz-Jeghers syndrome may be a premalignant condition. Consequently, even asymptomatic gastric, duodenal, and colonic polyps should be removed endoscopically. If surgical intervention is necessary, intraoperative endoscopy with polypectomy may prevent the development of a short bowel syndrome. Colonoscopic screening of patients and their family members may be beneficial and surveillance for extraintestinal malignancy appears to be warranted.