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加德纳综合征患者的诊断与外科治疗

The diagnosis and surgical treatment of patients with Gardner's syndrome.

作者信息

Watne A L, Lai H Y, Carrier J, Coppula W

出版信息

Surgery. 1977 Sep;82(3):327-33.

PMID:888098
Abstract

Of one hundred and twenty-six patients with Gardner's syndrome, 60% showed soft tissue tumors, 32% showed osteomatosis, 67% polyposis, and 20% the complete triad. Bowel cancer developed in 32% of the patients. The frequency of other diseases in these patients showed fibrous tumors in 8%, and two patients with cancer of the ampulla of Vater; otherwise the diseases seen did not show any major variation from what might be expected for the group at risk. Laboratory evaluation has included the demonstration of increased fecal cholesterol and primary bile acids in these patients. The recommended surgical treatment is colectomy and ileorectal anastomosis at a measured 12 cm level. This level of ileorectal anastomosis may be vital in giving a regression of rectal polyps, which was seen in 15 to 17 patients so treated. The conversion of an ileosigmoid to an ileorectal anastomosis resulted in polyp regression in one patient. The oral administration of ascorbic acid gave polyp regression in seven of 10 patients. There may be a possible relationship of fecal coprostanol and cholesterol levels and polyp regression.

摘要

在126例加德纳综合征患者中,60%出现软组织肿瘤,32%出现骨瘤病,67%出现息肉病,20%出现完整三联征。32%的患者发生了肠癌。这些患者中其他疾病的发生率为8%出现纤维瘤,还有2例患 Vater壶腹癌;除此之外,所见到的疾病与高危人群预期的情况相比没有任何重大差异。实验室评估包括证明这些患者粪便中胆固醇和初级胆汁酸增加。推荐的手术治疗是在12厘米处进行结肠切除术和回直肠吻合术。这种回直肠吻合水平对于使直肠息肉消退可能至关重要,在15至17例接受该治疗的患者中可见到息肉消退。将回乙状结肠吻合改为回直肠吻合使1例患者的息肉消退。口服抗坏血酸使10例患者中的7例息肉消退。粪便粪甾烷醇和胆固醇水平与息肉消退之间可能存在某种关系。

相似文献

1
The diagnosis and surgical treatment of patients with Gardner's syndrome.加德纳综合征患者的诊断与外科治疗
Surgery. 1977 Sep;82(3):327-33.
2
Gardner's syndrome: report of a family.加德纳综合征:一个家族的报告。
Ann Surg. 1974 Aug;180(2):198-202. doi: 10.1097/00000658-197408000-00012.
3
[Results of ileorectal or low ileosigmoid anastomosis after total or subtotal colectomy].[全结肠或次全结肠切除术后回直肠或低位回乙状结肠吻合术的结果]
Bull Acad Natl Med. 1968 Mar 19;152(11):173-81.
4
Surgical management of familial polyposis and Gardner's syndrome.家族性腺瘤性息肉病和加德纳综合征的外科治疗
Am J Surg. 1979 Jan;137(1):54-6. doi: 10.1016/0002-9610(79)90010-2.
5
Immediate outcome, long-term function and quality of life after extended colectomy with ileorectal or ileosigmoid anastomosis.回肠直肠或回肠乙状结肠吻合术扩大结肠切除术后的近期疗效、长期功能及生活质量
Colorectal Dis. 2014 Aug;16(8):O288-96. doi: 10.1111/codi.12558.
6
Effect of ascorbic acid on rectal polyps of patients with familial polyposis.抗坏血酸对家族性腺瘤性息肉病患者直肠息肉的影响。
Surgery. 1975 Nov;78(5):608-12.
7
Gardner's syndrome, diagnosis and treatment. Report on a family.加德纳综合征的诊断与治疗。一家系报告。
Acta Chir Scand. 1979;145(4):267-72.
8
Spontaneous regression of rectal polyps following abdominal colectomy and ileorectal anastomosis for familial adenomatous polyposis, without sulindac treatment: report of four cases.家族性腺瘤性息肉病患者行腹会阴联合切除术及回肠直肠吻合术后直肠息肉的自发消退,未接受舒林酸治疗:4例报告
Endoscopy. 2007 Jul;39(7):665-8. doi: 10.1055/s-2007-966643.
9
Colectomy with ileorectal anastomosis for familial adenomatous polyposis: the risk of rectal cancer.家族性腺瘤性息肉病行结肠切除术加回直肠吻合术:直肠癌风险
Surgery. 1987 Jan;101(1):20-6.
10
Long-term follow-up after colectomy and ileorectal anastomosis in familial adenomatous polyposis coli. Is there still a place for the procedure?家族性腺瘤性息肉病患者行结肠切除和回肠直肠吻合术后的长期随访。该手术仍有存在的价值吗?
Hepatogastroenterology. 1989 Apr;36(2):109-12.

引用本文的文献

1
Gardner's syndrome. Recent developments in research and management.加德纳综合征。研究与管理的最新进展。
Dig Dis Sci. 1980 Dec;25(12):945-59. doi: 10.1007/BF01308046.
2
The occurrence of carcinoma of the rectum following ileoproctostomy for familial polyposis.家族性息肉病行回肠直肠吻合术后发生直肠癌。
Ann Surg. 1983 May;197(5):550-4. doi: 10.1097/00000658-198305000-00008.
3
Familial colonic cancer syndromes.家族性结肠癌综合征
West J Med. 1983 Sep;139(3):351-9.