Grosfeld J L, West K W
Arch Surg. 1986 May;121(5):530-4. doi: 10.1001/archsurg.1986.01400050040005.
Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened.
5例患者（年龄范围为18个月至16岁）发生广泛性幼年性息肉病。临床表现包括腹痛、虚弱、直肠出血、腹泻、直肠脱垂、肠套叠、杵状指和发育不良。实验室检查结果包括贫血、低白蛋白血症、低钾血症和皮肤试验无反应性。通过双重对比灌肠、内镜检查和活检进行诊断。与孤立性幼年性息肉患者不同，广泛性受累的患者需要手术干预。次全结肠切除术和回肠直肠吻合术是首选术式，我们对4例患者实施了该手术。1例直肠疾病持续存在的患者需要进行回肠肛管-直肠拖出术。所有5例患者目前均存活且状况良好。由于息肉可能持续至成年期，长期随访很重要。家庭成员有发生胃肠道肿瘤的风险，应进行筛查。
