Surgical treatment of familial adenomatous polyposis in children: cross-sectional study

L. R. Khabibullina, A. Razumovsky, O. Sherbakova
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Abstract

Familial adenomatous polyposis (FAP) is an autosomal dominant disease caused by the APC gene mutation, characterized by colon adenomas and colorectal cancer, including in children. The issue of timing and indications for surgical treatment of FAP in childhood remains debatable.AIM: to identify predictors of surgery for FAP in pediatric patients.PATIENTS AND METHODS: a retrospective case-control study was conducted. The results of treatment of 50 children with FAP who were in the surgical department of the Russian Children's Clinical Hospital from January 2000 to April 2023 were analyzed. Two groups were formed: patients who underwent surgery of FAP under the age of 18 (case), and patients who did not undergo surgery at this age (control). We analyzed potential predictors: the age of manifestation, the clinical, the characteristics of adenomas, the anemia and family history, polyposis of the upper gastrointestinal tract.RESULTS: in the surgical group, the proportion of patients with more than 100 adenomas was higher (23 (88%) versus 11 (45%) (p=0.002)). It was revealed that the number of adenomas was more than 100 at the time of the first colonoscopy (OR 12 (95% CI (3–80), p=0.02) and the presence of colon bleeding (OR 5.8 (95% CI 1–35, p=0 .03) are independent predictors of colproctectomy in children.CONCLUSION: the number of adenomas over 100 and colon bleeding are independent predictors of colproctectomy in childhood.
儿童家族性腺瘤性息肉病的手术治疗:横断面研究
家族性腺瘤性息肉病(FAP)是由APC基因突变引起的常染色体显性疾病,以结肠腺瘤和结直肠癌为特征,包括儿童。儿童FAP手术治疗的时机和适应症仍有争议。目的:探讨小儿FAP手术的预测因素。患者和方法:进行回顾性病例对照研究。分析2000年1月至2023年4月在俄罗斯儿童临床医院外科收治的50例FAP患儿的治疗结果。分为两组:18岁以下行FAP手术的患者(病例)和18岁未行手术的患者(对照组)。我们分析了可能的预测因素:表现的年龄,临床,腺瘤的特征,贫血和家族史,上消化道息肉病。结果:在手术组中,超过100个腺瘤的患者比例更高(23例(88%)vs 11例(45%)(p=0.002))。结果显示,第一次结肠镜检查时腺瘤的数量超过100个(OR 12 (95% CI (3-80), p=0.02)和结肠出血的存在(OR 5.8 (95% CI 1-35, p= 0.03)是儿童结肠切除的独立预测因素。结论:100岁以上的腺瘤数目和结肠出血是儿童结肠直肠切除术的独立预测因素。
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